Dual Coronary-Pulmonary Artery Fistula in a Patient with Severe Bicuspid Aortic Valve Stenosis.

A 62-year-old male presented to the emergency department with acute viral bronchitis and worsening of his chronic dyspnea on exertion. Incidentally, a murmur was detected on physical examination. Extensive work-up, including coronary computed tomography angiography, revealed a rare combination and potential association between severe bicuspid aortic valve stenosis and coronary-pulmonary artery fistulas.

Long-term prognosis and predictors of outcomes after negative stress echocardiography.

Negative stress echocardiography (NSE) is associated with low cardiovascular morbidity and overall mortality. We aimed to determine the clinical and echocardiographic predictors of overall and cardiovascular outcomes following NSE. Patients who underwent SE between 2013 and 2017 were reviewed. Patients with a history of solid organ transplant or being evaluated for transplant, history of end-stage renal or liver disease, and positive SE were excluded. NSE results were divided into negative diagnostic if patient reached target heart rate (THR) and had no wall motion abnormality (WMA) at rest or stress; negative non-diagnostic if patient had no WMA but did not reach THR or if image quality was non-diagnostic; and abnormal non-ischemic if patient had a resting WMA not worsened at stress along with a personal history of coronary artery disease (CAD). New CAD lesion at 1 year was defined as ≥ 50% stenosis on cardiac catheterization. Of 4119 patients with SE, 2575 were included. All-cause mortality rate was 1.1%/year and CAD rate was 3.1%/year. Predictors of all-cause mortality were age, male gender, history of smoking and being selected for dobutamine SE. Predictors of a new CAD lesion at 1 year were male gender, diabetes, personal history of CAD and abnormal non-ischemic SE. We identified clinical and echocardiographic characteristics in a subset of NSE patients who are at higher risk for subsequent adverse events. These characteristics should be accounted for during the clinical interpretation of SE, and patients found at increased risk for morbidity and mortality warrant continued follow-up.

Performance of Traditional Pretest Probability Estimates in Stable Patients Undergoing Myocardial Perfusion Imaging.

BACKGROUND: The yield of myocardial perfusion imaging is low in contemporary patients with suspected coronary artery disease (CAD) selected based on American College of Cardiology Foundation/American Heart Association pretest probability estimate. We compared traditional pretest estimates of CAD probability with the prevalence of abnormal myocardial perfusion single-photon emission computed tomography (MPS). METHODS: This was a cohort study from a single academic center. Consecutive stable patients without known CAD referred for stress MPS for suspected CAD between 2004 and 2011 were identified (n=15 777). Angina typicality was determined using standard criteria. Abnormal MPS perfusion was defined as a summed stress score ≥4, ischemia as summed stress score ≥4 and summed difference score ≥2, and extensive ischemia as summed difference score ≥8 using a standard, 17-segment model of the left ventricle. The pretest probability of CAD was determined using the American College of Cardiology Foundation/American Heart Association criteria. RESULTS: Overall, 14% (n=2177) of patients had abnormal MPS of whom 11% (n=1698) had ischemia and 4% (n=684) extensive ischemia. In patients with chest pain who underwent treadmill MPS (n=4764), only 27% reported angina on the treadmill. Typical angina was associated with the highest prevalence for positive MPS (33% in men and 14% in women), ischemia (30% in men and 12% in women), and extensive ischemia (22% in men and 4% in women) when compared with other symptom categories. Prevalence of MPS abnormality was substantially lower than expected based on pretest probability estimates across most sex and age groups. In multivariable analysis, the pretest probability estimate was not an independent predictor of abnormal MPS. CONCLUSIONS: Traditional estimates of pretest probability of CAD are not predictive of MPS perfusion abnormality and overestimate its prevalence in stable patients.

False-positive stress echocardiograms: Predictors and prognostic relevance.

BACKGROUND: Recent studies indicate that the pretest likelihood of significant coronary artery disease (CAD) (≥50% luminal stenosis) is over-estimated and that the frequency and severity of positive stress tests have been decreasing. This suggests an increased prevalence of false-positive (FP) stress tests. The aims of this retrospective study were to investigate the predictors of FP stress echocardiography (SE) and to compare the outcomes of patients with FP results to those with true-positive (TP) results. METHODS: Patients who underwent SE between 2013 and 2017 in a tertiary-care center were reviewed. Included were patients aged ≥40years who had cardiac catheterization (CC) within 1year of the index stress test. SE was considered FP if a new or worsening wall motion abnormality was present in the absence of significant corresponding CAD. RESULTS: Of the 5100 patients with SE, 1069 satisfied inclusion criteria. A total of 305 patients had positive SE results; of which 162 (53%) were FP. Logistic regression revealed that female gender (p=0.009), the absence of diabetes (p=0.03), the absence of a personal history of CAD (p=0.004), and lower stress WMSI (p=0.03) were independently associated with FP results. Patients with FP results on SE had similar all-cause mortality to those with TP results. CONCLUSIONS: Accounting for predictors of FP findings on SE could improve the interpretation of SE results and limit the use of unnecessary CC. Furthermore, patients with FP results on SE could benefit from aggressive risk factor control and careful clinical follow-up.

Serum albumin concentration as an independent prognostic indicator in patients with pulmonary arterial hypertension.

BACKGROUND: Serum albumin is a strong prognostic indicator for many disease processes, yet limited data exist regarding its prognostic relationship in pulmonary arterial hypertension (PAH). Our study aims to assess the relationship of hypoalbuminemia with disease severity and mortality in this population. HYPOTHESIS: Serum albumin concentrations are a predictor of outcomes in PAH. METHODS: A retrospective review of all patients with World Health Organization group 1 PAH evaluated between March 2001 and August 2008 was performed. Patients were stratified into groups based on serum albumin concentration ≤3.3 g/dL (hypoalbuminemia) vs >3.3 g/dL. Clinical, hemodynamic, and survival comparisons were compared between groups using Student t test and χ2 test, followed by univariate analysis and multivariate logistic regression. RESULTS: A total of 163/273 (59.7%) patients had a documented serum albumin concentration. Hypoalbuminemia was present in 41 (25.2%) patients and serum albumin ≤3.3 g/dL represented the lowest quartile of serum albumin. Patients with hypoalbuminemia had higher rates of renal dysfunction (26.8% vs 9.8%, P =0.0069) and hepatic dysfunction (29.3% vs 6.6%, P <0.001), and lower hemoglobin levels (11.6 vs 13.4 g/dL, P < 0.001). Hemodynamic and functional capacity assessments were comparable between groups. Independent predictors of mortality included low albumin levels (hazard ratio [HR]: 0.485, P = 0.008), high right atrial systolic area (HR: 1.062, P = 0.003), low Fick-derived cardiac index (HR: 1.465, P = 0.016), and high New York Heart Association functional class (HR: 1.767, P = 0.042). Patients with hypoalbuminemia demonstrated a significantly lower survival rate at latest follow-up (P = 0.01). CONCLUSIONS: Lower serum albumin concentrations in patients with PAH are associated with higher mortality and can serve as a marker of disease severity in this patient population.

The prognostic significance of troponin I elevation in acute ischemic stroke.

BACKGROUND: The significance of cardiac troponin I (TnI) levels in patients with acute ischemic stroke remains unclear. METHODS: Data were prospectively collected on 1718 patients with acute ischemic stroke (2009-2010). Patients with positive TnI (peak TnI ≥0.1 µg/L) were assigned to the myocardial infarction (MI) group if they met diagnostic criteria. The remaining patients with positive TnI were assigned to the no-MI group. Patients were followed up for 1.4 ± 1.1 years. Primary outcome was inhospital and long-term all-cause mortality. RESULTS: Positive TnI was present in 309 patients (18%), 119 of whom (39%) were classified as having MI. Positive TnI was independently associated with older age, hypertension, smoking, peripheral arterial disease, heart failure, higher systolic blood pressure, higher serum creatinine, and lower heart rate (P < .01). Patients with MI had the highest inpatient mortality (P < .001) and the lowest survival rate by Kaplan-Meier analysis (P < .0001). Peak TnI greater than or equal to 0.5 µg/L, particularly if satisfying criteria for MI, was independently associated with long-term mortality (P < .0001); peak TnI less than 0.5 µg/L alone was not when adjusted for covariates. CONCLUSION: Positive TnI greater than or equal to 0.5 µg/L in patients with acute ischemic stroke was independently associated with worse outcomes. Patients with diagnosis of MI represent a particularly high-risk subgroup.

Prognostic Value of Pericardial Effusion on Serial Echocardiograms in Pulmonary Arterial Hypertension.

BACKGROUND: Pericardial effusion in pulmonary arterial hypertension (PAH) is an indicator of right heart failure and poor prognosis; its significance on serial transthoracic echocardiograms (TTE) is not clear. METHODS: Baseline and follow-up TTE (1.0 ± 0.5 years), clinical parameters, and outcomes were studied (N = 200) in consecutive patients with PAH who underwent TTE at our center between October 1999 and November 2007. Study baseline TTE was 2.8 ± 4.0 years from initial PAH diagnosis. RESULTS: Over median follow-up of 3.6 ± 2.6 years from baseline TTE, 106 patients (53%) died. Pericardial effusion was present in 20% at baseline, and at any time during the study in 29%. Patients with any pericardial effusion during follow-up were more likely to have underlying connective tissue disease. They also had significantly higher mean right atrial pressure and pulmonary vascular resistance, had lower cardiac output by invasive hemodynamic studies, had higher serum creatinine, and were more likely to be treated with prostanoids. Patients were also significantly likely to have more echocardiographic right atrial dilation and right ventricular dilation and dysfunction, and worse tricuspid regurgitation with higher peak velocity. During follow-up, there was significantly increased use of prostanoids (58% vs. 28%) and combination therapy (8% vs. 2%) compared to baseline. Persistence of pericardial effusion on both baseline and follow-up TTE was associated with worse outcome, and an independent predictor of survival after adjusting for age, creatinine, functional class, and hemodynamics (P < 0.01). CONCLUSION: Persistence of pericardial effusion in PAH despite vasoactive therapy predicts worse outcomes; absence or resolution of pericardial effusion with therapy suggests better prognosis.

Outcomes after endocarditis or device infection in patients with left ventricular epicardial leads versus coronary sinus leads.

BACKGROUND: There is scant data about outcomes in patients with left ventricular epicardial (LVE) leads who develop endocarditis or device-related infection. OBJECTIVE: This retrospective study evaluated mortality and recurrence of infection among patients with LVE leads in comparison to patients with endovascular coronary sinus (CS) leads after the development of endocarditis or device-related infection. METHODS: Patients with cardiac resynchronization therapy (CRT) devices who developed endocarditis or pocket infection over 5 years at Cleveland Clinic were included in the study. The groups were all patients with LVE leads versus CRT devices without epicardial leads that developed endocarditis or pocket infection. Mortality was assessed using the Social Security Death Index and re-infection was assessed by reviews of the medical record. RESULTS: Prospective extraction of the CRT device and leads occurred among all 50 patients with CS leads and 8 of the 14 patients with LVE leads. The survival rate was 92.9 versus 92 % and freedom from re-infection rate was 64.3 versus 80 % in the patients with LVE leads versus CS leads, respectively, over 1 year (P value = 0.918 and 0.226, respectively). At 3 years, the survival rate in LVE lead group was 92.9 % and freedom from re-infection rate was 64.3 % in comparison to survival rate of 90 % and freedom from re-infection rate of 68 % in the CS group (P value = 0.751 and 0.798, respectively). CONCLUSION: After development of endocarditis or pocket infection, no statistically significant differences were seen in mortality, or recurrent infection between patients with LVE leads and those with CS leads.

Echocardiographic sizing of the left atrium of the transplanted heart.

BACKGROUND: The left atrium (LA) in the transplanted heart (TH) is a surgically created chamber from variable portions of donor and recipient LA. The American Society of Echocardiography (ASE) recommends measuring LA size in native hearts using biplane LA volume index (LAVI) as a more accurate measurement than LA anterior-posterior diameter (LA-APD). We hypothesized that LA-APD underestimates LA size when compared with LAVI in the TH. METHODS: Patients with a TH from 1/05 to 1/10 who had a transthoracic echocardiogram within 24 months of transplant were studied. Patients were excluded if they had an ejection fraction <50%, left ventricular hypertrophy, >mild aortic or mitral regurgitation, or poor image quality. Echocardiograms were analyzed for LA-APD and LAVI. LA size was classified as normal, mild, moderate, or severely enlarged based on age and sex specific cutoffs per the ASE. RESULTS: Of 297 patients evaluated, 160 met inclusion criteria. Mean age was 57 ± 13 years and 79% were males. Mean LA-APD and LAVI were 4.3 ± 0.6 cm and 36 ± 14 mL/m(2), respectively. LAVI correlated with LA-APD (r = 0.43, R(2) = 0.18, P < 0.0001). By LA-APD measurement, 20 (13%) LA were classified as having moderate or severe LA enlargement versus 80 (50%) by LAVI, P = 0.001. LA size was underclassified by ≥ 2 classes in 44 (28%) by LA-APD versus LAVI. CONCLUSIONS: In the setting of cardiac transplant, the LA size classified by LA-APD is commonly less than that classified by LAVI.

Effect of obesity on B-type natriuretic peptide levels in patients with pulmonary arterial hypertension.

Brain natriuretic peptide (BNP) levels are lower in obese patients with left ventricular failure than in their comparably ill, leaner counterparts. The effect of obesity on BNP in patients with pulmonary arterial hypertension (PAH) is unknown. We reviewed our prospective PAH registry data collected from November 2001 to December 2007 for patients undergoing right heart catheterization who met the criteria for PAH and had the BNP level and body mass index determined at baseline. The median BNP level for the lean, overweight, and obese patients was 285 pg/ml (interquartile range 131 to 548), 315 pg/ml (interquartile range 88 to 531), and 117 pg/ml (interquartile range 58 to 270), respectively (p = 0.029). A greater body mass index was associated with a lower BNP level, adjusted for age, gender, New York Heart Association functional class, hypertension, coronary artery disease, and mean right atrial and pulmonary arterial pressures (p <0.001). No statistically significant differences were found among the groups in age, race, medical co-morbidities, underlying etiology of PAH, use of vasoactive medications, New York Heart Association functional class, echocardiographic parameters, or pulmonary function. Obese patients had greater right atrial and pulmonary artery pressures. Increased BNP was associated with worse survival in the lean and overweight patients only. In conclusion, the BNP levels are attenuated in obese patients with PAH despite similar or worse hemodynamics or functional class compared to lean or overweight patients and should therefore be interpreted with caution.

Comparison of baseline predictors of prognosis in pulmonary arterial hypertension in patients surviving ≤2 years and those surviving ≥5 years after baseline right-sided cardiac catheterization.

Idiopathic pulmonary arterial hypertension (PAH) is usually associated with a poor outcome but the prognosis with other forms of PAH is not well-described. Advances in therapy have furthered clouded the disease course. We sought to determine the baseline indicators of prognosis in patients with PAH. We reviewed the records of patients with PAH followed up at our institution to identify those who died within 2 years (reduced survival group; n = 21) and those who survived >5 years (long survival group; n = 60). The groups were compared for prognostic significance of the baseline clinical parameters. The reduced survival group were older (p = 0.001) and more likely to have scleroderma-associated PAH (p = 0.01), have pericardial effusion (p = 0.01), have a shorter 6-minute walk test (6MWT) distance (p = 0.001), to require oxygen during 6MWT (p = 0.02), have a worse World Health Organization functional class (p <0.001), and have greater serum brain natriuretic peptide levels (p = 0.01). Regression analysis showed age, World Health Organization functional class, 6MWT distance, the need for oxygen during the 6MWT, and renal disease to be independently associated with a poor prognosis. In conclusion, age, PAH etiology, World Health Organization functional class, pericardial effusion, 6MWT distance, the need for oxygen during the 6MWT, and brain natriuretic peptide are predictors of prognosis in patients PAH receiving specific therapy and might help identify a group that could benefit from aggressive upfront therapy.

Sleep quality, depression, and quality of life in patients with pulmonary hypertension.

Pulmonary hypertension is a disabling disease characterized by progressive functional worsening, right heart failure, and death. Although pulmonary hypertension has been associated with poor quality of life, sleep quality has not been investigated in pulmonary hypertension patients. This was a cross-sectional study in which patients (N = 40) were asked to complete standardized questionnaires to assess sleep quality [using Pittsburgh Sleep Quality Index (PSQI)], insomnia, sleepiness, dyspnea, depression, restless leg syndrome, and quality of life [using Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR)] during routine office visits. Baseline hemodynamics, pulmonary function tests, exercise capacity, and transthoracic echocardiogram were analyzed. Pulmonary hypertension functional class was World Health Organization class II [20 (50%)], III [18 (45%)], and IV [2 (5%)], and 29 (72.5%) had poor sleep quality. PSQI score was associated with CAMPHOR symptoms score (R = 0.61, P < 0.001), CAMPHOR activities score (R = 0.38, P = 0.016), CAMPHOR quality-of-life score (R = 0.45, P = 0.004), depression (R = 0.42, P = 0.007), and dyspnea (R = 0.36, P = 0.02). Sleep quality was not associated with age, gender, other comorbidities, pulmonary hypertension etiology, baseline hemodynamics, pulmonary function testing, echocardiographic parameters, or exercise capacity. Poor sleep quality is common in patients with pulmonary hypertension and correlates with depression, dyspnea, and poor quality of life. Improving sleep quality in patients with pulmonary hypertension may improve quality of life.

Left atrial epicardial adiposity and atrial fibrillation.

BACKGROUND: Atrial fibrillation (AF) has been linked to inflammatory factors and obesity. Epicardial fat is a source of several inflammatory mediators related to the development of coronary artery disease. We hypothesized that periatrial fat may have a similar role in the development of AF. METHODS AND RESULTS: Left atrium (LA) epicardial fat pad thickness was measured in consecutive cardiac CT angiograms performed for coronary artery disease or AF. Patients were grouped by AF burden: no (n=73), paroxysmal (n=60), or persistent (n=36) AF. In a short-axis view at the mid LA, periatrial epicardial fat thickness was measured at the esophagus (LA-ESO), main pulmonary artery, and thoracic aorta; retrosternal fat was measured in axial view (right coronary ostium level). LA area was determined in the 4-chamber view. LA-ESO fat was thicker in patients with persistent AF versus paroxysmal AF (P=0.011) or no AF (P=0.003). LA area was larger in patients with persistent AF than paroxysmal AF (P=0.004) or without AF (P<0.001). LA-ESO was a significant predictor of AF burden even after adjusting for age, body mass index, and LA area (odds ratio, 5.30; 95% confidence interval, 1.39 to 20.24; P=0.015). A propensity score-adjusted multivariable logistic regression that included age, body mass index, LA area, and comorbidities was also performed and the relationship remained statistically significant (P=0.008). CONCLUSIONS: Increased posterior LA fat thickness appears to be associated with AF burden independent of age, body mass index, or LA area. Further studies are necessary to examine cause and effect, and if inflammatory, paracrine mediators explain this association.

Radiofrequency ablation of atrial fibrillation under therapeutic international normalized ratio: a safe and efficacious periprocedural anticoagulation strategy.

BACKGROUND: The best periprocedural anticoagulation strategy at the time of pulmonary vein isolation (PVI) is not known. Most centers stop administering warfarin (Coumadin) and use bridging with heparin or enoxaparin. OBJECTIVE: The purpose of this study was to evaluate the efficacy and safety of PVI under therapeutic international normalized ratio (INR). METHODS: Between January 2005 and December 2008, PVI was performed in 3,052 patients with therapeutic INR (> or =1.8) at the time of ablation. All patients were evaluated for ischemic strokes and bleeding complications. RESULTS: Mean INR was 2.53 +/- 0.62. Only 3 (0.098%) patients had ischemic strokes. One patient had a hemorrhagic stroke on the third day postablation but recovered completely by 1-week follow-up. Bleeding complications occurred in 34 (1.11%) patients; most were minor (0.79%). Major hemorrhagic complications occurred in 10 (0.33%) patients (tamponade in 5, hematomas requiring intervention in 2, transfusion necessary in 3). CONCLUSION: In a large patient population, continuation of Coumadin at a therapeutic INR at the time of PVI without use of heparin or enoxaparin for bridging is a safe and efficacious periprocedural anticoagulation strategy. It is an acceptable and potentially better alternative to strategies that use bridging with heparin or enoxaparin.

Thyroid acropachy.

Thyroid acropachy is a rare complication of thyroid disease most often occurring in patients with a long history of active Graves' disease with ophthalmopathy and dermopathy already present. It presents with digital clubbing and soft tissue swelling with possible pain in the digits. Plain radiographs show a solid periosteal reaction that tends to be bilateral and generally symmetrical involving the tubular bones of the hands and feet. Treatment is directed at the thyroid dysfunction and the accompanying ophthalmopathy and dermopathy. It is important to recognize the association with a more severe ophthalmopathy. In the absence of Graves' ophthalmopathy and dermopathy, a diligent search for other causes of acropachy is warranted.